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[size=20px]A 10-year-old boy undergoing chemotherapy for acute lymphoblastic leukemia develops right lower quadrant pain, tenderness, fever and tachycardia. Computed tomography (CT) scanning demonstrates a thickened appendix, cecum and ascending colon with pericecal inflammation. A complete blood count (CBC) reveals an absolute neutrophil count of < 500. The next best step in treatment is:

 

a Broad spectrum antibiotics

 

b Recombinant human granulocyte-colony stimulating factor (GCSF)

 

c Plasmapheresis

 

d Laparoscopic appendectomy

 

e Open right hemicolectomy[/size]

  • Author

Correct answer

a Broad spectrum antibiotics

 

Although there are no uniformly endorsed diagnostic criteria, the terms neutropenic enterocolitis, neutropenic enteropathy and typhlitis are used interchangeably to describe the occurrence of fever, abdominal pain and frequently diarrhea in children undergoing chemotherapy for malignancy. In most series, the commonest associated malignancies are hematologic, most commonly ALL, followed by AML and Burkitt’s lymphoma. It is estimated that approximately 8% of patients with acute leukemia will develop neutropenic enterocolitis. Although less frequent, it has also been described in association with most solid tumors including Ewing sarcoma, Wilms tumor, neuroblastoma, rhabdomyosarcoma and brain tumors. Most patients will have received chemotherapy in the previous two weeks, although the condition has been described prior to the initiation of chemotherapy.

 

 

Approximately half of all patients are neutropenic (ANC< 500) at diagnosis. Although many studies rely on radiologic findings as diagnostic criteria, there are no strict radiologic definitions. Mural thickening >3 mm involving colon and/or small bowel by ultrasound or CT scan has been suggested as diagnostic and patients with mural thickening of >10mm face a potentially higher mortality rate. Acute appendicitis does occur in oncology patients in association with neutropenia and should be differentiated from neutropenic enterocolitis. A recent study from the Pediatric Surgical Oncology Research Collaborative which relied on diagnostic confirmation by ultrasound, CT or MRI, reported 66 patients treated with either up front appendectomy or antibiotics with equivalent outcomes. Patients with cecal or colonic inflammation were considered to have neutropenic enterocolitis and were excluded.

 

 

Broad spectrum antibiotics are the mainstay of treatment, and surgery is reserved for complications, usually perforation or hemorrhage which occur infrequently. GCSF use is usually restricted to patients with prolonged neutropenia in settings where the primary goal is avoidance of chemotherapy delay. As such, there are no specific recommendations for its immediate use in neutropenic enterocolitis. Mortality in neutropenic enterocolitis is usually < 10% and is most common in patients presenting with septic shock.

 

 

There is no role for plasmapheresis in the treatment of neutropenic enterocolitis.

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